What is tet spell and how is it treated?
The principle objectives during the management of Tet spell are to decrease catecholamine production, increase blood oxygenation, increase systemic vascular resistance, reduce right ventricular outflow obstruction and increase pulmonary blood flow.
How are cyanotic spells (tet spells) treated?
Conventional treatment of cyanotic spells or Tet spells is sedation with morphine, keeping in knee chest position, oxygen administration, hydration and intravenous propranolol. Knee chest position compresses the femoral arteries and veins, thereby enhancing the afterload and reducing return of desaturated blood from the lower limbs.
What causes tet spells in children?
TET Spells. TET spells occur most often in children who have a congenital heart defect called Tetralogy of Fallot. Children with Tetralogy of Fallot have a combination of heart problems. A ventricular septal defect (VSD), which is a hole between the two lower chambers of the heart.
What are the signs and symptoms of tet spells?
A ventricular septal defect (VSD), which is a hole between the two lower chambers of the heart. Pulmonary stenosis (PS), which is a narrowing at or beneath the pulmonary valve. Overriding aorta, in which the aorta lies directly above the VSD. Thickening of the right ventricular wall. Children having a TET spell may have several symptoms:
What is the treatment for a tet spell?
The pathophysiology of a tet spell is a decrease in systemic vascular resistance, increasing a right to left shunt which leads to a bunch more things that all just keep increasing the right to left shunt. The key to treatment is to increase systemic vascular resistance.
Why does knee to chest help tet spells?
Talk to your doctor about how you can manage your child's tet spells. Your doctor may suggest that you: Bring the child's knees up tight against his or her chest (this is called the knee-chest position) or have your child squat down. This will increase blood flow to the lungs.
What are tet spells?
Tet spells Sometimes, babies who have tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying or feeding, or when agitated. These episodes are called tet spells. Tet spells are caused by a rapid drop in the amount of oxygen in the blood.
How does morphine help with tet spells?
If the hypercyanotic spell is ongoing give intravenous or intramuscular (if no cannula) morphine. This helps calm the child down, reduces tachypnea and decreases pulmonary vascular resistance.
What is the knee-chest position used for?
Introduction: The knee-chest (KC) position is often used for spine surgery. It is considered to promote significant changes in venous return and cardiac output. However, the magnitude of these changes and their consequences on intraoperative haemodynamics and anaesthetic requirements remain to be determined.
What is tet spell in tetralogy of Fallot?
Infants with tetralogy of Fallot can have a bluish-looking skin color―called cyanosis―because their blood doesn't carry enough oxygen. At birth, infants might not have blue-looking skin, but later might develop sudden episodes of bluish skin during crying or feeding. These episodes are called tet spells.
Is a tet spell an emergency?
Tet spell is an episodic central cyanosis due to total occlusion of right ventricle outflow in a patient with a congenital heart disease, such as Tetralogy of Fallot (TOF). There are limited cases of patients who lived with untreated TOF until adulthood. Tet spell in an adult is rarely seen in an emergency department.
What is the medical use of morphine?
Morphine injection is used to relieve moderate to severe pain. It may also be used before or during surgery with an anesthetic (medicine that puts you to sleep). Morphine belongs to the group of medicines called narcotic analgesics (pain medicines). It acts on the central nervous system (CNS) to relieve pain.
How long do tet spells last?
Severe attacks may lead to the loss consciousness, and occasionally to convulsions or temporary paralysis on one side of the body (hemiparesis). These spells may last for a few minutes to a few hours and may be followed by periods of muscle weakness and a prolonged period of sleep.
How does knee chest position increase systemic vascular resistance?
The knee–chest position may also increase SVR by reducing arterial blood flow to the lower extremities. 2. Morphine sulfate suppresses the respiratory center and abolishes hyperpnea.
What is the tet spell?
The 'Tet spell' (also called 'hypoxic spell', 'cyanotic spell', 'hypercyanotic spell' or 'paroxysmal dyspnea') most frequently occurs in young infants with Tetralogy of Fallot but may occur with other congenital heart defects that have pulmonary or subpulmonary stenosis and a VSD, and at any age. Common precipitants include crying, defecation, ...
What is a hypercyanotic spell?
Hypercyanotic spells may be best thought of as an imbalance between pulmonary and systemic vascular resistance favouring decreased pulmonary flow and increased right-to-left sh unting. Hypoxemia, metabolic acidosis, hyperpnoea, increased systemic venous return, catecholamines, and pulmonary vasoconstriction are thought to be involved in an interaction that results in a self-perpetuating cycle. Infundibular spasm is not required.
Is Fallot a self-limiting spell?
While medical intervention is indicated, many episodes are self-limiting. Those involved in the care of a child with Tetralogy of Fallot should be familiar with a spell's presentation and early management.
What is a hypercyanotic spell?
Hypercyanotic spells are sudden episodes of profound cyanosis and hypoxia that may be triggered by a fall in oxygen saturation (eg, during crying, defecating), decreased systemic vascular resistance (eg, during playing, kicking legs), or sudden tachycardia or hypovolemia.
What is a ventricular septal defect?
The ventricular septal defect in tetralogy of Fallot is often described as a malalignment type, since the conal septum is displaced anteriorly. This displaced septum protrudes into the pulmonary outflow tract, often resulting in obstruction and hypoplasia of the downstream structures, including the pulmonary valve, main pulmonary artery, and branch pulmonary arteries. The ventricular septal defect is typically large; thus, systolic pressures in the right and left ventricles (and in the aorta) are the same. Pathophysiology depends on the degree of right ventricular outflow obstruction. A mild obstruction may result in a net left-to-right shunt through the VSD; a severe obstruction causes a right-to-left shunt, resulting in low systemic arterial saturation (cyanosis) that is unresponsive to supplemental oxygen.
How rare are Tet spells?
The incidence of Tet spell peaks between the second month and second year of life and reduces in frequency after that [2]. Tet spells are exceedingly rare in adults [3]. A typical Tet spell is triggered by a cry, with progressive tachypnea, deep breathing, progressing to worsening of cyanosis, and if not corrected, may end as syncope, convulsions, cerebrovascular accident or rarely death [1-4]. Other triggers are the stress of feeding, waking up from a long deep sleep, fever, dehydration, defecation, anaesthetic agents, cardiac catheterization or rarely supraventricular tachycardia[1-6]. The oxygen saturation is lower than the child’s usual levels, and the murmur of right ventricular outflow obstruction softens or disappears.
What is a tetralogy of fallot?
Tetralogy of Fallot is the most common cyanotic congenital heart disease. Tet spell, also called a hyper cyanotic spell, hypoxic spell, or cyanotic spell, is a hallmark clinical manifestation of Fallot physiology characterized by a paroxysmal episode of hypoxia due to reduction in pulmonary blood flow [1]. Although typical of Tetralogy of Fallot, other congenital heart diseases with Fallot physiology like double outlet right ventricle with pulmonary stenosis, pulmonary atresia with ventricular septal defect, tricuspid atresia with pulmonary stenosis, and transposition of great arteries with ventricular septal defect and pulmonary stenosis can develop cyanotic spells. With time, the morbidity and mortality associated with Tetralogy of Fallout have improved markedly owing to early detection, corrective surgery and medical care.
How much morphine should I take for infundibular spasm?
Morphine should be given at the dose of 0.1mg/kg to 0.2 mg/kg by intravenous route. The intramuscular or subcutaneous route can be used if intravenous access is not available. Some reports suggest use of midazolam, dexmedetomidine and fentanyl [11-13].
What to do if no improvement is observed?
If no improvement is observed, the child needs to be immediately shifted to a hospital facility. Supplemental oxygen should be administered using a face mask or nasal cannula. This increases the oxygen content of the blood and reduces the pulmonary vascular resistance. Meanwhile, intravenous access has to be obtained for further interventions.
Hypercyanotic Spell Basics
Paroxysmal hypoxemic events, that can lead to quite heroically low oxygen saturations (today the kid in the ED had an initial sat of 20 – that’s on the “low-ish” side I think).
Management of Hypercyanotic Spell – Decrease PVR, Increase SVR, Improve PBF
Knee-Chest Position (increases Preload and increases SVR) Can even compress abdominal aorta to increase SVR more.
What causes tet spells?
patients often present with tet spells caused by crying, fever, or any physical exertion. acute onset of restlessness causes increased cyanosis, gasping, and occasionally syncope. tet spells often resolve with knee-chest position, oxygen, or morphine. usual onset is around 2-6 months of age. Physical exam.
What is the tetralogy of Fallot?
Tetralogy of Fallot. A neonatal boy is found to be cyanotic a few hours after delivery. The mother noticed the neonate turning blue when crying and feeding. The boy was born via a spontaneous vaginal delivery without any complications and to a 30-year-old mother.